title: Recurrent venous thrombosis in Ehlers-Danlos syndrome type III: an atypical manifestation.
abstract: Ehlers-Danlos syndrome (EDS) comprises a group of hereditary connective tissue disorders in which collagen synthesis and fibrogenesis are impaired. Patients with EDS type III have a bleeding tendency manifested by ecchymoses and haematomas. However, thrombotic events are rare in this entity. Herein, we present a 48-year-old Hispanic man with EDS type III who had recurrent cephalic vein thrombophlebitis and thrombosis, and brachial vein thrombosis. Tests for hypercoagulable disorders including antithrombin III activity, protein C activity, protein S activity, anticardiolipin antibodies, homocysteine levels, factor V Leiden mutation and prothrombin gene mutation were negative. The patient required long-term anticoagulation with warfarin. After 3 years follow-up, he did not present further thrombotic events. Clinicians should be aware that patients with EDS might be at risk for hypercoagulable disorders.
authors.affiliation: Department of Medicine, Division of Rheumatology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico.
authors.forename: Esther
authors.initials: E
authors.lastname: Jiménez-Encarnación
authors.forename: Luis M
authors.initials: LM
authors.lastname: Vilá
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use*; D001132:Arm / Q000098:blood supply*; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D055502:Secondary Prevention; D013924:Thrombophlebitis / Q000209:etiology; D020246:Venous Thrombosis / Q000209:etiology* / Q000517:prevention & control; D014859:Warfarin / Q000627:therapeutic use*
chemical_list: D000925:Anticoagulants; D014859:Warfarin